Metastatic Ameloblastoma Research Articles

Difference in survival and prognosis between malignant tumors of odontogenic origin

IntroductionThe prognosis of metastatic ameloblastoma (METAM), now defined as a benign tumor, and malignant odontogenic tumors (MOT) is poorly studied in the literature. The aim of this study was to determine the prognosis and factors influencing the survival of these patients. Material and methodsUsing the SEER database, we retrieved clinical data of patients with malignant tumors of dental origin between 1975 and 2020. They include 3 histological groups: metastatic ameloblastoma (METAM), malignant odontogenic tumors (MOT, including odontogenic carcinoma, odontogenic sarcoma, primary intraosseous carcinoma, and ameloblastic carcinoma) and ameloblastic fibrosarcoma (AFS). ResultsOn 251 patients, we observed no significant difference in disease-specific survival (DSS) between the three histological groups, with 5-year DSS of 77.4% (59.9 – 100), 84.0% (78.7 – 89.7) and 71.1% (50.7 – 99.7) for METAM, MOT and AFS respectively (p = 0.460). In the MOT group, using a multivariate analysis, surgical treatment appeared to be a protective factor for DSS (HR = 0.483 [0.243 - 0.960], p = 0.038) after taking into account the potential confounding factors mentioned above. No variable significantly influenced DSS in the METAM and AFS group in univariate analysis. DiscussionWe found no difference in specific survival between the three histological groups. Although metastatic ameloblastoma was reclassified as a benign tumor by the WHO in 2017, the vital risk for patients remains consequent, and statistically similar to malignant odontogenic tumors.

Anti-MAPK Targeted Therapy for Ameloblastoma: Case Report with a Systematic Review.

Ameloblastoma, a benign yet aggressive odontogenic tumor known for its recurrence and the severe morbidity from radical surgeries, may benefit from advancements in targeted therapy. We present a case of a 15-year-old girl with ameloblastoma successfully treated with targeted therapy and review the literature with this question: Is anti-MAPK targeted therapy safe and effective for treating ameloblastoma? This systematic review was registered in PROSPERO, adhered to PRISMA guidelines, and searched multiple databases up to December 2023, identifying 13 relevant studies out of 647 records, covering 23 patients treated with MAPK inhibitor therapies. The results were promising as nearly all patients showed a positive treatment response, with four achieving complete radiological remission and others showing substantial reductions in primary, recurrent, and metastatic ameloblastoma sizes. Side effects were mostly mild to moderate. This study presents anti-MAPK therapy as a significant shift from invasive surgical treatments, potentially enhancing life quality and clinical outcomes by offering a less invasive yet effective treatment alternative. This approach could signify a breakthrough in managing this challenging tumor, emphasizing the need for further research into molecular-targeted therapies.

The Role of BRAF Inhibitors in the Management of Ameloblastoma: A Literature Review.

Ameloblastoma is one of the most prevalent but enigmatic benign odontogenic tumors of the jaw, accounting for approximately 10% of all maxillary and mandibular tumors. This neoplasia is distinguished by exhibiting several clinical and histological variants along with several mutations that affect its behavior. The ameloblastoma treatment plan is determined by the tumor's size, anatomical location, histologic variant, and anatomical involvement. On chromosome 7, there is a proto-oncogene called BRAF. When BRAF is mutated, it becomes an oncogene and continuously produces proteins like MEK and ERK, members of mitogen-activated protein kinase (MAPK). In the signaling pathway, these proteins activate transcription factor inside the nucleus that helps in cell division and growth. Numerous neoplasms have been linked to more than 40 BRAF mutations. The most common one is BRAF proto-oncogene serine/threonine kinase (BRAF) V600E, whose treatment has been linked to a positive outcome. BRAF inhibitors like vemurafenib, dabrafenib, and sorafenib have shown excellent results, especially in metastatic ameloblastoma. BRAF, particularly in the case of metastatic ameloblastoma, inhibitors such as vemurafenib, dabrafenib, and sorafenib, has demonstrated outstanding results. Targeted therapies have been employed as adjuvant therapies to enhance cosmetic outcomes, even though no reports of serial cases demonstrate their effectiveness in ameloblastomas. In the treatment of ameloblastomas, the identification of BRAF V600E and additional mutations as the prime targeted therapies has proven to be a significant breakthrough where surgical treatment was contraindicated. In this article, we review the presence of BRAF V600E mutations, their inhibitors, and targeted therapies in ameloblastoma.

BRAF-targeted therapy for locally advanced ameloblastoma of the mandible: A potential neoadjuvant strategy.

3149 Background: Ameloblastoma is a rare benign but locally aggressive odontogenic neoplasm, with 2% of cases representing ameloblastic carcinoma or metastatic ameloblastoma. It affects young adults with high recurrence rates after surgery. The standard therapy is radical bone resection with subsequent functional, aesthetic & psychological impairments. Therefore, other therapeutic options, including neoadjuvant approach, should be considered. Sixty to 70% of mandible Ameloblastoma carry a BRAF mutation, usually V600E, and previous case reports have shown durable responses to treatment with BRAF inhibitors in these patients. We sought to explore the possibility of neoadjuvant BRAF or BRAF+MEK inhibition as neoadjuvant treatment in mandible Ameloblastoma. Here we present results of 12 patients with locally advanced disease who were treated with BRAF with or without MEK inhibitors. Methods: Patients who were unable to undergo jaw preservation surgery for locally advanced Ameloblastoma with a BRAF V600E mutation were treated with Dabrafenib or Dabrafenib-Tratmetinib in an EAP form. Patient records were analyzed for baseline parameters, treatment regimen, toxicity, response to therapy and the ability to convert to a mandible preservation surgery. Data were collected and analyzed in accordance with Sheba Medical Center IRB approval. Statistical analyses were done with STATA v.17. Results: Twelve patients were treated with Dabrafenib/ Dabrafenib-Tratmetinib between 2017-2021. Five patients received BRAF-MEK inhibitors and 7 BRAF inhibitor alone. Median age was 21. Ten patients(83%) showed excellent response to therapy and have successfully converted from planned radical bone resection to mandible preservation surgery. The other 2 patients are still on therapy and have also showed deep responses that enable conversion to mandible preservation. Median time to surgery was 10 months. With median follow up of 18 months, no cases of recurrence were documented. Rate of adverse events was as expected with only 1 case of G3-4 (hepatitis). Conclusions: Targeted therapy with BRAF with or without MEK inhibition may serve as an important therapeutic tool for locally advanced Ameloblastoma with the potential of organ preservation treatment, and is an important example of oncological therapy assisting in non-cancerous tumors.

Metastatic ameloblastoma with postoperative accelerated tumor growth treated with carboplatin and paclitaxel: a case report

Ameloblastoma is a histologically benign odontogenic epithelial tumor that rarely metastasizes. However, metastasis to the lungs can occur, usually years after the development of the primary tumor. Here, we present the case of a 63-year-old woman with metastatic ameloblastoma in the lungs that developed 12 years after surgery for the primary lesion. As is typical for metastatic ameloblastomas, the tumor was incidentally found on radiography and surgically removed. However, the tumor exhibited accelerated progression with pleural metastasis after surgical removal, which is unusual in metastatic ameloblastoma. The patient was successfully treated with carboplatin/paclitaxel and showed a partial response to tumor progression, implying that this approach can be safely used in the absence of a standard treatment regimen.

An unusual biphasic pulmonary lesion: late metastasis from a mandibular ameloblastoma

Ameloblastoma is mostly a benign odontogenic tumour most frequently arising within the mandible and maxilla. Although ameloblastoma can be locally aggressive and prone to local recurrence, metastasis is a rare (and usually late) complication. We report a case of a metastatic ameloblastoma to the lung following an original presentation with a traumatic jaw fracture through what was thought to be a mandibular odontogenic cyst in 1988. The definitive diagnosis of ameloblastoma was not reached until a local recurrence occurred ten years later; this was managed with hemimandibulectomy and post-operative radiotherapy. A single lung lesion was identified incidentally 33 years after the original presentation: it demonstrated typical morphological features of ameloblastoma and FOXP1 immunostaining was confirmatory. Recent advances in understanding the genetic pathogenesis of ameloblastoma demonstrate frequent BRAFV600E mutations as well as mutations in other MAP kinase pathway genes such as KRAS, NRAS, HRAS and FGFR2.1 Several case reports have indicated promising results with BRAF inhibitor therapy in metastatic ameloblastoma.2 1.Brown N, Betz B. Ameloblastoma: a review of recent molecular pathogenetic discoveries. Biomark Cancer 2015; 7 (Suppl 2): 19–24.2.González-González R, López-Verdín S, Lavalle-Carrasco J, et al. Current concepts in ameloblastoma-targeted therapies in B-raf proto-oncogene serine/threonine kinase V600E mutation: Systematic review. World J Clin Oncol 2020; 11: 31–42.

Maxillary ameloblastoma with lung metastasis: Case report of a rare tumor

Ameloblastomas are low-grade neoplasms of odontogenic epithelium that account for about 1% of all oral tumors and about 10% of odontogenic tumors. Rarely, these tumors have a potential of distance metastasis, and once they do metastasize, they are termed as metastatic ameloblastoma. This article aims at describing a case of a rare form of maxillary ameloblastoma that metastasized to the lung and challenges in its management in settings with limited resources. A 24-year-old African male presented with a massive swelling on the right side of the face for 2 years in 2018. He was previously operated on for a swelling on the right side of the maxilla in 2012, which was histologically diagnosed as ameloblastoma. Clinical and radiological evaluation revealed a massive maxillary tumor that had a local extension to the brain. X-ray and computed tomography (CT) scan of chest pointed out to metastatic mass in the lungs. Histology of the recurrent tumor mass revealed it to be follicular ameloblastoma, and CT-guided fine-needle aspiration cytology of the lung mass showed microscopic features of ameloblastoma similar to the primary jaw tumor.

Características Clínico-Patológicas e Tratamento do Ameloblastoma Unicístico: Relato de Caso

The ameloblastoma is an epithelial benign odontogenic tumor which is locally invasive, slow-growing and presents considerable recidive rates. It is considered the most clinically relevant benign odontogenic tumor, currently being classified as “ameloblastoma”, “unicystic ameloblastoma”, “peripheral or extraosseous ameloblastoma” and “metastatic ameloblastoma”. Unicystic type is the second most prevalent ameloblastoma, occurring in approximately 15% of all ameloblastomas, showing specific characteristics, especially in younger patients. The aim of this study is to clarify the characteristics of this type of ameloblastoma, presenting the case report of a young patient affected by the tumor in the jaw, highlighting clinical, imaginological, histopathological, therapeutic and prognostic aspects involved. In this case, the chosen treatment was, initially, the marsupialization of the lesion and posteriorly the enucleation with peripheral ostectomy and tooth extraction involved. This treatment option has shown good results, decreasing the patients damage when properly indicated, for this, an early and assertive diagnosis is essential, associating clinical and histopathological characteristics. The patient keeps on careful follow-up, since the proliferation pattern of the mural type requires greater attention given its greater possibility of recidive.

Persistent response to vemurafenib in metastatic ameloblastoma with BRAF mutation: a case report

BackgroundAmeloblastomas are uncommon locally aggressive tumors of odontogenic epithelium that rarely metastasize. Currently, there is no standard of care for the metastatic forms. Several studies have shown that ameloblastomas frequently have a BRAF mutation.Case presentationWe report a case of a 33-year-old Caucasian woman with ameloblastoma diagnosed 30 years ago who developed lung metastasis 19 years ago. Systemic oral treatment with vemurafenib, a BRAF inhibitor, was initiated 28 months ago within the AcSé French basket clinical trial of vemurafenib.ConclusionsThe patient has shown a durable clinical, functional, and radiographic partial response with vemurafenib. These observations suggest the possibility of introducing neoadjuvant and/or adjuvant targeted therapy in locally advanced ameloblastoma to improve outcome. BRAF inhibition has proved to be an efficient strategy in patients with a BRAF-mutated ameloblastoma.

Malignant ameloblastoma: multiple local recurrence and metastasis in the scalp. Case report

Introduction: Ameloblastoma is a neoplasm usually found in the maxilla and mandible with progressive intraosseous growth and a tendency to local recurrence. Malignant or metastatic ameloblastoma is one of its rare variants; a histopathological study is required for diagnosis confirmation. The common sites of dissemination are the lungs and cervical lymph nodes. The treatment is surgical and radiotherapy or chemotherapy are palliative options.Case report: A case of malignant ameloblastoma was reported in a 39-year-old man with metastasis to the scalp and multiple local recurrences. The patient required mandibular osteotomy, multiple local resections, partial maxillary resection, free flap reconstruction, local scalp resection and free flap coverage. Due to a new relapse that could not be treated with a surgical approach, radiotherapy was ordered.Discussion: The site most frequently affected by malignant ameloblastoma due to hematogenous dissemination is the lung. This paper reports the case of a patient with compromised scalp. To date, according to a search conducted, only one case report about this type of metastatic neoplasm has been published.Conclusion: Histopathological diagnosis of ameloblastoma associated with surgical resection of the primary tumor and the metastatic tumor is the only approach that can offer disease-free survival.

Pulmonary Metastasis of Mandibular Amelobastoma: A Case Report

SESSION TITLE: Lung Cancer 2 SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Tuesday, October 31, 2017 at 07:30 AM - 08:30 AM INTRODUCTION: Ameloblastoma is an odontogenic tumor originating from the enamel of the teeth. Although benign, local recurrent tissue growth can be aggressive. While distant metastasis to the lung is rare, early diagnosis can translate into effective treatment implementation. CASE PRESENTATION: A 72 year old male presented to the emergency department twelve years after undergoing mandibulectomy for mandibular amelobastoma. This benign neoplasm did recur once locally for which he underwent definitive treatment with radiation therapy. He presented with complaints of sudden onset chest pain that was pleuritic and non-radiating. The pain lasted thirty minutes and dissipated spontaneously. He had a non-productive cough but denied any hemoptysis or constitutional symptoms. Patient was vitally stable with an unremarkable physical exam. Pertinent imaging is shown below. Ultimately, bronchoscopy was performed and a large, exophytic endobronchial lesion of the left lower lobe was biopsied. Histopathological staining pattern revealed cells consistent with metastatic ameloblastoma. DISCUSSION: Metastasis is the active or passive dissemination of neoplastic disease from site of origin to distant organs, usually due to a malignant condition. Ameloblastoma is a benign tumor with locally aggressive behavior that can also metastasize. This occurs after an interval ranging from 10-12 years and the lungs are involved in 75-80% of cases. Histopathologically, these tumors have well-differentiated, benign features but are clinically metastatic. The mechanism for metastatic spread remains debatable. Seeding from surgical manipulation of the primary tumor, in its odontogenic form, suggests that aspiration may contribute. However, the presence of tumor cells in surrounding blood vessels suggests hematogenous spread of disease. Intense 18-fluorodeoxyglucose (FDG) activity seen on positron emission tomography (PET) scan may reflect the aggressiveness of the disease. CONCLUSIONS: Pulmonary ameloblastoma is a rare metastatic neoplasm that is not necessarily malignant. Its radiologic and histopatholgical features aid in attaining correct diagnosis and ultimately lead physicians towards an appropriate treatment plan. Reference #1: Bi et al. Maligant ameloblastoma in the lung: 3 cases of misdiagnosis as primary lung tumor with a unique growth pattern. Diagnostic Pathology (2015) 10:123-129. Reference #2: Rabo et al. Mandibular Ameloblastoma with Lung Metastasis 10 Years after Resection. Philipp J Otolaryngol head Neck Surg. (2016); 31(1):53-56 DISCLOSURE: The following authors have nothing to disclose: Kevin Charles, Ashley Ferraro, Michael Constantinescu, Adil Yousuf No Product/Research Disclosure Information

Mandibular Ameloblastoma with Lung Metastasis 10 Years after Resection

Objective: To present a case of mandibular ameloblastoma with pulmonary metastasis after ten years and discuss the possible pathophysiology, diagnostic and therapeutic options.
 Methods:
 Study Design: Case Report
 Setting: Tertiary Private Hospital
 Subject: One
 Conclusion: Though benign, ameloblastoma has a high propensity for local invasion and may metastasize. It is difficult to predict metastasis, even with adequate treatment of the primary lesion. There is no standard protocol to prevent or detect metastatic ameloblastoma, but regular and close follow up may ensure early diagnosis.
 Keywords: ameloblastoma, metastatic ameloblastoma, lung metastasis, follicular type ameloblastoma, odontogenic tumor

Multiple nodular lung metastases with no obvious primary showing low positive 18-fluoro deoxyglucose (FDG) uptake on positron emission tomography (PET) scan: A unique case of metastatic ameloblastoma

Abstract Introduction The incidental detection of multiple secondaries in the lung, in an asymptomatic individual calls for detailed evaluation, especially if the 18-fluoro-deoxyglucose positron emission tomography (FDG PET) scan results are inconclusive or unusual. Presentation of case A 45 year old lady, asymptomatic, presented to us for evaluation of incidentally diagnosed, bilateral pulmonary metastases with a provisional diagnoses of advanced lung cancer. FDG PET scanning was inconclusive and revealed low positive FDG uptake. The biopsy revealed it to be metastatic ameloblastoma. She refused surgery but took only one cycle of chemotherapy with paclitaxel and carboplatin, which she then subsequently discontinued but continues to be asymptomatic at last followup. Discussion She gave history of previous ameloblastoma of the left mandible 13 years back, and recurrences 3 and 6 years later, which had been resected. For the last seven years she had no evidence of disease. History of past illness including operative interventions done for apparently benign or locally aggressive lesions of the oral cavity such as an ameloblastoma is important in evaluation of such cases. Conclusion Pulmonary metastases arising from an ameloblastoma maybe associated with low FDG positive uptake, and even though they are rare, it is important to diagnose them, since the prognosis and management of lesions from lung or other primaries and those from ameloblastoma are markedly different.

Metastatic ameloblastoma to a cervical lymph node: a case report and review of literature

Case presentation A 54-year-old man, who 3 years prior had undergone successful surgical treatment of a left mandibular ameloblastoma, presented with a left submandibular swelling. Radiology confirmed a heterogenous solid nodule close to the previous mandibular resection site and the soft tissue nodule was resected. Histology revealed a lymph node containing a deposit of tumour with features typical of ameloblastoma. These included anastomosing strands of cells, peripheral palisading with reverse nuclear polarity and a dense fibrotic stroma. A further radical left neck dissection had 21 lymph nodes, none of which contained metastatic tumour. Discussion Ameloblastoma is a rare benign odontogenic tumour with locally aggressive behaviour and a high recurrence rate. Despite its benign histological appearance, ameloblastoma has the ability to metastasise, which occurs in less than 1% of cases. Most metastatic deposits are seen in the lung with few reported cases of lymph node metastases. Due to the rarity of cases, treatment of ameloblastoma with metastases to lymph nodes is controversial. Some recommend radical neck dissection, while others have proposed simple excision with close follow up in cases with a single node metastasis.

Ameloblastic carcinoma: An analysis of 12 cases with a review of the literature.

The diagnosis of ameloblastic carcinoma is often difficult and the optimal treatment methods remain controversial. The current study retrospectively investigated the optimal diagnosis and treatment methods of 12 ameloblastic carcinoma patients at the West China Hospital of Stomatology, Sichuan University (Chengdu, China), and 20 patients selected from the PubMed database, were reviewed. The clinical features, diagnosis and outcome of the different treatments were evaluated. Ameloblastic carcinoma occurred in 12 out of a total of 538 ameloblastoma patients; the majority were of the primary type. Of the 538 ameloblastoma patients, 294 were male, 244 were female with a male to female ratio of 1.2:1. The predilection age is 20–30 years, which accounts for 40% of the total. In total, 461 cases were in the mandible and 77 were located in the maxilla. The cure rate of the primary type and the recurrence rate of the secondary type tumors were higher in the patients from the West China Hospital of Stomatology compared with those reported in the literature. In particular, a case with a long-term survival of 30 years is presented, which is considered to be relatively rare. The evolution of the clinical course has experienced three stages: Ameloblastoma (1978) followed by metastatic ameloblastoma (2000) and finally ameloblastic carcinoma (2008). To avoid recurrence, wide local excision with postoperative radiation therapy is required. While novel therapeutic regimens should also be considered as appropriate, including carbon ion therapy and Gamma Knife stereotactic radiosurgery. However, controlled studies with larger groups of patients are required to increase the accuracy of results.

Metastasizing Ameloblastoma – A perennial pathological enigma? Report of a case and review of literature

The Ameloblastoma is a slow growing locally invasive odontogenic epithelial neoplasm with a high recurrence rate and a low tendency to metastasize. Metastasis in Ameloblastoma was first described by Simmons and Emura in the 1920s. Slootweg and Muller proposed the term Malignant Ameloblastoma to describe a well-differentiated ameloblastoma that metastasizes but maintains the characteristic cytologic features of the original tumour and the term Ameloblastic Carcinoma to an ameloblastoma with malignant cytological features. About 2% of ameloblastomas undergo metastasis. So far there have only been two cases of Metastasizing Ameloblastoma reported from the Indian Subcontinent. We present the case of a 22-year-old male Indian patient, who presented with a diffuse swelling in the left posterior mandible. Radiographs revealed a multilocular radiolucency in the left mandible. On histopathological examination, the lesion was diagnosed as follicular ameloblastoma. Four years later the patient presented with a swelling in the left submandibular region. Histological examination revealed metastatic ameloblastoma within the cervical lymph node.

Ameloblastoma with varied sites of metastasis: Report of two cases and literature review

ObjectiveWe report two rare cases of lung metastasis from maxillary ameloblastoma, in order to review its risk and analyse the types of metastases that can present with this disease. MethodsA 40-year-old male with multiple recurrences and a 46-year-old female, who had undergone successful surgical treatment of a maxillary ameloblastoma, presented with metastatic lesions. The primary tumour and metastases were benign in both patients. We reviewed and analysed 20 cases of the same condition reported in recent years. ResultsOur initial treatment for the primary maxillary lesion was performed more than 10 years before the pulmonary lesions presented. Due to the aggressive nature of this tumour, metastases in the lungs and cervical lymph nodes (male patient) were confirmed. ConclusionThese cases presented a diagnostic challenge due to the multiple and varied sites of recurrence, which indicate the natural behaviour of this tumour. Different routes of metastasis can occur, including implanting, haematogenous, and lymphatic spread. CT-guided percutaneous transthoracic lung biopsy is an important method to confirm metastatic ameloblastoma.

Multifocal Large Cystic Liver Metastasis of a Sinonasal Tract Ameloblastoma: An Unusual Clinical Presentation

Ameloblastoma is a slowly growing, locally invasive tumor of odontogenic epithelial origin. On rare occasion, a well-differentiated ameloblastoma may metastasize. Recently, we encountered a unique case of metastatic ameloblastoma in the liver in a 48-year-old woman. Preoperative radiographic study revealed multiple cystic lesions in the liver, 6 of which were confirmed intraoperatively. Macroscopic examination of the resected tumors revealed well-circumscribed, encapsulated cystic lesions. Sections from the tumors demonstrated solid interlocking islands with peripheral palisading of “basaloid cells” and squamous differentiation of centrally located cells. The cytology of these tumors was bland and the mitotic activity was low. Immunohistochemistry showed immunoreactivity for CK5/6 and p63, immunoreactivity for CD10 in the peripherally palisading cells and immunoreactivity for CK19 in the centrally located cells. The resected liver cystic tumors were originally diagnosed as “low-grade cystic ameloblastoma-like tumor in the liver” and suggested a possibility of metastasis. The patient was doing well clinically after the resection of her liver lesions. However, CT scan of the head and neck region at another hospital one year later showed an enhancing, expansile, well circumscribed mass in the right nasal cavity. Biopsy and resection of the nasal mass revealed an ameloblastoma invading the maxillary bone. The patient is doing well 2 months after her nasal surgery. To our knowledge, this is the first case of ameloblastoma manifested as liver metastases prior to the identification of a sinonasal primary. J Med Cases. 2013;4(8):544-549 doi: https://doi.org/10.4021/jmc1314w

Multifocal Large Cystic Liver Metastasis of a Sinonasal Tract Ameloblastoma: An Unusual Clinical Presentation

Ameloblastoma is a slowly growing, locally invasive tumor of odontogenic epithelial origin. On rare occasion, a well-differentiated ameloblastoma may metastasize. Recently, we encountered a unique case of metastatic ameloblastoma in the liver in a 48-year-old woman. Preoperative radiographic study revealed multiple cystic lesions in the liver, 6 of which were confirmed intraoperatively. Macroscopic examination of the resected tumors revealed well-circumscribed, encapsulated cystic lesions. Sections from the tumors demonstrated solid interlocking islands with peripheral palisading of “basaloid cells” and squamous differentiation of centrally located cells. The cytology of these tumors was bland and the mitotic activity was low. Immunohistochemistry showed immunoreactivity for CK5/6 and p63, immunoreactivity for CD10 in the peripherally palisading cells and immunoreactivity for CK19 in the centrally located cells. The resected liver cystic tumors were originally diagnosed as “low-grade cystic ameloblastoma-like tumor in the liver” and suggested a possibility of metastasis. The patient was doing well clinically after the resection of her liver lesions. However, CT scan of the head and neck region at another hospital one year later showed an enhancing, expansile, well circumscribed mass in the right nasal cavity. Biopsy and resection of the nasal mass revealed an ameloblastoma invading the maxillary bone. The patient is doing well 2 months after her nasal surgery. To our knowledge, this is the first case of ameloblastoma manifested as liver metastases prior to the identification of a sinonasal primary. J Med Cases. 2013;4(8):544-549 doi: https://doi.org/10.4021/jmc1314w

Metastatic ameloblastoma responding to combination chemotherapy: case report and review of the literature

BackgroundAmeloblastoma is a rare benign odontogenic tumor with locally aggressive behavior and a high recurrence rate. When metastases occur, which are uncommon, lungs constitute the most frequent site involved. Malignant ameloblastomas are different from ameloblastic carcinomas. Malignant ameloblastomas are tumors considered metastatic despite the appearance of well-differentiated or benign histology, while ameloblastic carcinomas are histologically malignant in both primary and metastatic sites.Case presentationA 24-year-old Moroccan man presented a malignant ameloblastoma of the mandible. The tumor was entirely resected. Five years later, a local recurrence occurred. Our patient was treated by exclusive radiotherapy with persistence of a residual disease. After two years he developed multiple lung metastases. Our patient received a combination chemotherapy using doxorubicin and cisplatin.ConclusionLess than 50 cases of ameloblastoma with metastases have been reported. There is still no standard treatment for metastatic ameloblastoma. Only through continuous reporting of such cases will clinicians be able to draw an optimal strategy for management of this pathology.